Related Experiment Video

Updated: Jun 23, 2026

Isolation of Human Lymphatic Endothelial Cells by Multi-parameter Fluorescence-activated Cell Sorting
07:36

Isolation of Human Lymphatic Endothelial Cells by Multi-parameter Fluorescence-activated Cell Sorting

Published on: May 1, 2015

A vasculopathy with deposition of lambda light chain crystals

G C Stone1, B A Wall, I R Oppliger

  • 1University of Washington, Seattle.

Annals of Internal Medicine
|February 15, 1989
PubMed

Insights

This study reports two fatal cases of obstructive vasculopathy linked to lambda light chain deposits. These rare conditions, mimicking vasculitis, showed no inflammation and were unresponsive to standard treatments.

Area of Science:

  • Nephrology
  • Hematology
  • Pathology

Background:

  • Systemic necrotizing vasculitis is an inflammatory condition affecting blood vessels.
  • Monoclonal gammopathies involve abnormal production of immunoglobulins by a single clone of B cells.
  • Light chain deposition disease can lead to organ damage, but crystalline deposits causing obstructive vasculopathy are rare.

Purpose of the Study:

  • To describe two unique cases of fatal obstructive vasculopathy.
  • To investigate the role of monoclonal lambda light chains in the pathogenesis of these vascular lesions.
  • To differentiate this condition from typical systemic necrotizing vasculitis.

Main Methods:

  • Clinical case presentation of two patients with subacute obstructive vasculopathy.
  • Serum protein electrophoresis and immunofixation to detect monoclonal proteins.
  • Tissue biopsy and crystal analysis to identify the composition of deposits.

Main Results:

  • Both patients presented with obstructive vasculopathy and extensive crystalline tissue deposits.
  • Monoclonal lambda light chain components were identified in the serum of both patients.
  • Cryocrystalglobulinemia with lambda light chain dimers was confirmed in one patient; inflammation was histologically absent.
  • Treatment with corticosteroids, cyclophosphamide, and plasmapheresis did not alter the rapid clinical deterioration.

Conclusions:

  • Monoclonal lambda light chain deposition can cause a distinct, rapidly fatal obstructive vasculopathy.
  • This condition is clinically and histologically distinguishable from systemic necrotizing vasculitis.
  • The lack of response to immunosuppressive and plasmapheresis therapies highlights the unique nature of this light chain-mediated vascular injury.

More Related Videos

Three-Dimensional Imaging of the Vertebral Lymphatic Vasculature and Drainage using iDISCO+ and Light Sheet Fluorescence Microscopy10:05

Three-Dimensional Imaging of the Vertebral Lymphatic Vasculature and Drainage using iDISCO+ and Light Sheet Fluorescence Microscopy

Published on: May 22, 2020

Analyses of Proteinuria, Renal Infiltration of Leukocytes, and Renal Deposition of Proteins in Lupus-prone MRL/lpr Mice09:43

Analyses of Proteinuria, Renal Infiltration of Leukocytes, and Renal Deposition of Proteins in Lupus-prone MRL/lpr Mice

Published on: June 8, 2022

Related Concept Videos

Type IV Collagen of Basal Lamina01:05

Type IV Collagen of Basal Lamina

Type IV collagen is a 400 nm long, network-forming collagen that acts as a barrier between the epithelial and endothelial cells. Type IV collagen  forms the backbone of the basement membrane by scaffolding with laminin, entactin, proteoglycans, and fibronectin. Apart from rendering structural support to the basement membrane, it also helps entail signaling potentials necessary for both pathological and physiological functions.
A type IV collagen molecule has six alpha chains which can exist in...
Renal Corpuscle01:20

Renal Corpuscle

The glomerulus and Bowman's capsule are two essential components of the nephron, which is the functional unit of the kidney. These microscopic structures play a critical role in the process of blood filtration to produce urine.
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous capillaries...
Cardiomyopathy IV: Restrictive Cardiomyopathy01:29

Cardiomyopathy IV: Restrictive Cardiomyopathy

Restrictive cardiomyopathy (RCM) is a rare heart muscle disease characterized by impaired ventricular filling due to stiffened ventricular walls, leading to significant diastolic dysfunction.EtiologyRestrictive cardiomyopathy can arise from both inherited and acquired diseases, many of which are systemic. It is categorized into four main types: infiltrative, storage, non-infiltrative, and endomyocardial diseases.Infiltrative diseases, such as amyloidosis, lead to RCM by depositing amyloid...
Peripheral Artery Disease I: Introduction01:30

Peripheral Artery Disease I: Introduction

Peripheral artery disease (PAD) predominantly results from atherosclerosis, which involves the accumulation of fatty deposits, or plaques, within the walls of arteries. This causes them to narrow and harden, significantly reducing blood flow. PAD predominantly affects the legs, particularly the arteries supplying the thighs and calves. In rare cases, it may involve other arteries, including those in the arms.Etiology of PAD:The principal cause of PAD is atherosclerosis, which results from fatty...
Venous Thrombosis I: Introduction01:30

Venous Thrombosis I: Introduction

Venous thrombosis, the most common disorder of the veins, involves the formation of a thrombus or blood clot associated with vein inflammation. It can be classified as either superficial vein thrombosis or deep vein thrombosis.Superficial Vein Thrombosis: This involves the formation of a thrombus in a superficial vein, usually the greater or lesser saphenous vein. Though less severe than deep vein thrombosis (DVT), SVT can lead to complications if untreated.Deep Vein Thrombosis (DVT): This...
Nephrotic Syndrome I : Introduction01:24

Nephrotic Syndrome I : Introduction

Nephrotic Syndrome is a chronic kidney disorder defined by clinical findings such as severe proteinuria, hypoalbuminemia, hyperlipidemia, and edema. These symptoms result from damage to the glomeruli, the kidney’s filtering units, increasing their permeability to proteins.Definition and Meaning:Proteinuria, defined as the loss of more than 3.5 grams of protein per day in adults, is a crucial feature of nephrotic syndrome. This condition is often accompanied by edema, the accumulation of fluid...